Secondary Systemic Amyloidosis Research Articles

Beyond the spine: a case of ankylosing spondylitis complicated by secondary amyloidosis

BackgroundAnkylosing spondylitis (AS) is a chronic inflammatory condition primarily affecting the spine and sacroiliac joints, often associated with human leukocyte antigen B27 (HLA-B27) positivity. While musculoskeletal symptoms are typical manifestations, AS can also lead to systemic complications, including secondary systemic amyloidosis (SSA), also known as Amyloid A (AA) amyloidosis, involving multiple organs.Case presentationWe present a case of a 45-year-old Asian male with a complex medical history, including diabetes and hypertension, who developed AS complicated by SSA. The patient exhibited a diverse range of symptoms, including cardiac, renal, gastrointestinal, and musculoskeletal manifestations. He reported shortness of breath on exertion, orthopnea, pedal edema, generalized weakness, back pain, neck pain, low-grade fever, decreased appetite, frothy urine, and significant weight loss over the past year. Diagnostic evaluations revealed HLA-B27 positivity and histologically confirmed AA amyloidosis, providing a comprehensive understanding of the systemic involvement.ConclusionThis case report highlights the intricate interplay between AS and SSA, particularly AA amyloidosis, with a focus on its systemic impact beyond musculoskeletal symptoms. The tragic outcome, marked by severe cardiac involvement, underscores the challenges in managing such complex cases. This report emphasizes the importance of early diagnosis and treatment of AS to prevent severe complications, along with vigilant monitoring and individualized treatment plans, as well as the need for further research to enhance our understanding and improve management strategies for AS-related amyloidosis.

A Rare Case of Secondary Systemic Amyloidosis in a Young Patient with Crohn's Disease: A Case Report

A Rare Case of Secondary Systemic Amyloidosis in a Young Patient with Crohn's Disease: A Case Report

Ankylosing spondylitis status and risk of secondary systemic amyloidosis: A two-sample mendelian randomization study

ObjectivesThere is still controversy regarding the causal relationship between ankylosing spondylitis (AS) and secondary systemic amyloidosis (SSA). This study utilized aggregated data from genome-wide association studies (GWAS) on population cohorts to investigate whether a causal relationship exists between AS and SSA. MethodsThe genetic causal relationship between AS status and SSA was analyzed utilizing a two-sample Mendelian randomization (TSMR). The analyses were conducted using the weighted mode method (WM2), inverse variance weighted method (IVW), simple mode (SM), weighted median method (WM1), and Mendelian randomization Egger regression (MR-Egger). Among these methods, the primary results were based on the IVW approach. The association was evaluated using the odds ratio (OR) along with a 95% confidence interval (95% CI). ResultsThe IVW analysis revealed a positive causal relationship between AS status and SSA (OR = 1.411, 95 % CI = 1.069, 1.862, P = 0.015). Meanwhile, the WM1 (OR = 1.394, 95 % CI = 1.115, 1.742, P = 0.004) and WM2 (OR = 1.393, 95 % CI = 1.112, 1.743, P = 0.045) methods also identified a positive causal relationship between AS status and SSA. The MR-Egger method did not identify a causal relationship between AS and SSA (OR = 1.175, 95 % CI = 0.888, 1.555, P = 0.342). The SM results demonstrated that the observed genotypes did not exhibit statistically significant differences between AS and SSA (OR = 1.184, 95 % CI = 0.416, 3.366, P = 0.767). The results of the MR-Egger regression suggested that the results were unaffected by bias caused by genetic pleiotropy (Intercept = 0.283, SE = 0.134, P = 0.126). Cochran's Q test did not reveal any significant heterogeneity (Q = 1.759, P = 0.624). The “leave-one-out” analysis further confirmed that the absence of any single SNP did not impact the robustness of our results. ConclusionThis study revealed a positive causal relationship between AS status and the occurrence of SSA, providing new insights into the genetic analysis of SSA.

Unveiling Alarming Trends: Worsening All Cause Mortality in US Adults over 25 with Amyloid 1999-2020 Analysis

Introduction: Amyloidosis is a collective term for conditions with an abnormal extracellular deposition of amyloid protein. Amyloidosis can be classified based on the location of deposition (localized vs systemic) and by the different subtypes of proteins which are created as a result of a variety of diseases (plasma cell dyscrasias, chronic inflammation, nephrotic syndrome, genetic). Depending on the affected organs, amyloidosis can present with, not limited to, hepatomegaly, renal impairment, nerve conduction impairment, cardiac conduction abnormalities, restrictive cardiomyopathy, and multiorgan failure. In the last century there has been an increase in innovations leading to a better understanding of the pathology, diagnostic tests, staging, and treatment options for amyloidosis. Our study aims to compare all cause mortality trends in adults over 25 years old diagnosed with amyloidosis in the United States. Methods: The national mortality trends from 1999 to 2020 for all subtypes of amyloid were analyzed from the CDC WONDER database. The data was extracted using ICD codes E85 (Amyloidosis) with subgroups E85.0 (Non-neuropathic heredofamilial amyloidosis), E85.3 (Secondary systemic amyloidosis), E85.4 (Organ-limited amyloidosis), E85.8 (Other amyloidosis), and E85.9 (Amyloidosis, unspecified. Data was stratified for age groups above 25 years old. Age-adjusted mortality rates (AAMRs) were calculated per 100,000 people and death certificate data was stratified by reported sex, race/ethnicity, and US census region. Results: Between 1999 and 2020 there were 40,015 deaths from amyloid. Our analysis revealed that all-cause AAMR per 100,000 people with amyloid showed an increasing trend between 1999 to 2020 (Figure 1). Subgroup analysis on race/ethnicity revealed the AAMR for the Black population has been consistently higher to other groups (Table 1). On average, from 1999 to 2020, the Black population had the highest AAMR (1.52 AAMR) followed by the White population (0.78 AAMR), Hispanic (0.55 AAMR), and Asian (0.5 AAMR). Further subgroup analysis revealed men have a higher AAMR (1.9 AAMR, 2020) compared to women (0.8 AAMR, 2020). Overall there are similar census region trends of all mortality in Amyloid: North (1.5 AAMR, 2020), Midwest (1.5 AAMR, 2020), South (1.0, AAMR, 2020) and West (1.3 AAMR, 2020). Conclusion: Due to advancement in technology, amyloid diseases are becoming more widely recognized and increasingly diagnosed. Unfortunately, treatment of amyloidosis remains limited to early diagnosis and treatment of the underlying condition. This includes modalities such as chemotherapy and stem cell transplantation for plasma cell dysplasias and TTR stabilizers for hereditary amyloidosis. These trends in increase in AAMR likely reflect the increased awareness/diagnostics of amyloid disease and complications. The African American population may have higher AAMR due to socioeconomic and environmental factors, health disparities, and research bias. We also suspect men have a higher ATTR than women as it has been reported that ATTR and AL Amyloidosis tends to occur more frequently in men than women and is associated with more severe disease (Kroi et al., 2020, Desport et al., 2012). Further research is necessary to expand on the disparities seen with the ultimate goal of providing targeted therapies to reverse this downward trend.

Epidemiology of Amyloidosis and Genetic Pathways to Diagnosis and Typing

We reviewed our studies on epidemiology and germline genetics of amyloidosis. In epidemiology, we considered both hereditary and non-hereditary amyloidosis. As the source of data, we used the nationwide Swedish hospital discharge register. We estimated the incidence of hereditary ATTR amyloidosis, for which Sweden is a global endemic area, at 2/million. Surprisingly, the disease was also endemic within Sweden; the incidence in the province with the highest incidence was 100 times higher than in the rest of Sweden. Risk of non-Hodgkin lymphoma increased five-fold in the affected individuals. Among non-hereditary amyloidosis, the incidence for AL amyloidosis (abbreviated as AL) was estimated at 3.2/million, with a median survival time of 3 years. Secondary systemic amyloidosis (most likely AA amyloidosis) showed an incidence of 1.15/million for combined sexes. The female rate was two times higher than the male rate, probably relating to the higher female prevalence of rheumatoid arthritis. The median survival time was 4 years. We also identified patients who likely had familial autoinflammatory disease, characterized by early onset and immigrant background from the Eastern Mediterranean area. Young Syrian descendants had the highest incidence rate, which was over 500 times higher than that in individuals with Swedish parents. Germline genetics focused on AL on which we carried out a genome-wide association study (GWAS) in three AL cohorts (N = 1129) from Germany, UK, and Italy. Single nucleotide polymorphisms (SNPs) at 10 loci showed evidence of an association at p < 10−5; some of these were previously documented to influence multiple myeloma (MM) risk, including the SNP at the IRF4 binding site. In AL, SNP rs9344 at the splice site of cyclin D1, influencing translocation (11;14), reached the highest significance, p = 7.80 × 10−11; the SNP was only marginally significant in MM. The locus close to gene SMARCD3, involved in chromatin remodeling, was also significant. These data provide evidence for common genetic susceptibility to AL and MM. We continued by analyzing genetic associations in nine clinical profiles, characterized by organ involvement or Ig profiles. The light chain only (LCO) profile associated with the SNP at the splice site of cyclin D1 with p = 1.99 × 10−12. Even for the other profiles, distinct genetic associations were found. It was concluded that the strong association of rs9344 with LCO and t(11;14) amyloidosis offer attractive mechanistic clues to AL causation. Mendelian randomization analysis identified associations of AL with increased blood monocyte counts and the tumor necrosis factor receptor superfamily member 17 (TNFRSF17 alias BCMA) protein. Two other associations with the TNFRSF members were found. We discuss the corollaries of the findings with the recent success of treating t(11;14) AL with a novel drug venetoclax, and the application of BCMA as the common target of plasma cell immunotherapies.

Porcelain Heart: A Case of Diffuse Myocardial Calcification.

The pathophysiology of myocardial calcifications is variable and may have potentially fatal consequences if undiagnosed and untreated.

Among Commercially Available Fruit Juices, Pomegranate Is the Most Effective Inhibitor of PMS-Trypsin Amyloid-Like Fibrils Formation

The formation of amyloid fibrils is associated with many human illnesses, such as Alzheimer’s, Huntington’s, and Parkinson’s diseases, amyotrophic lateral sclerosis, spongiform encephalitis, type 2 diabetes, and primary and secondary systemic amyloidosis. Nutrition contributes to the prevention of these diseases. The aim of our work was to look for commercially available fruit juices that can inhibit the formation of amyloid fibrils. Of the fruit juices that we examined, that of pomegranate was found to be the most effective inhibitory agent using turbidity measurements and Congo red binding assay. According to our experiments, pomegranate juice reduced the amount of PMS-trypsin amyloid-like fibrils to 3.7% at 5-fold dilution compared with the sample without pomegranate. The inhibitory effect of the pomegranate juice was concentration dependent.

P-091 Patients with Inflammatory Bowel Disease Who Develop Secondary Systemic Amyloidosis Have Increased In-Hospital Mortality

Background:Systemic AA amyloidosis has been reported as a rare but severe complication of Inflammatory Bowel disease (IBD), particularly of Crohn's disease (CD). It is characterized by extracellular deposition of fibrillar proteins in organs leading to organ dysfunction. The aim of this study is to

Secondary systemic amyloidosis in inflammatory bowel disease: a nationwide analysis.

BackgroundSecondary systemic amyloidosis (SSA) is a rare but severe complication of inflammatory bowel disease (IBD). We aimed to evaluate the clinical characteristics, predictors of complications, and in-hospital mortality of patients with Crohn’s disease (CD) and Ulcerative colitis (UC) who develop SSA.MethodsUsing the National Inpatient Sample, we identified patients hospitalized for IBD and SSA between 2004 and 2012. Using multivariate logistic regression, patients with CD were compared with those with UC regarding the presence or absence of SSA. IBD patients without SSA were matched in a 2:1 ratio with those with SSA using propensity matching. We analyzed the hospitalization trends of SSA in CD and UC patients using Pearson’s χ2 test. Analyses were performed using SAS version 9.3.ResultsAmong the 302,548 patients with CD and 174,057 patients with UC hospitalized between 2004 and 2012, we identified 47 (0.02%) and 36 (0.02%) cases of SSA, respectively. We noted rising annual hospitalization trends for both CD and UC patients with or without SSA. In-hospital mortality was significantly higher for both the UC+SSA group (16.7% vs. 2.1%, P<0.0001) and the CD+SSA group (6.4% vs. 1.0%, P=0.0001) before propensity matching. However, this difference was not seen for either UC+SSA (17.1% vs. 7.1%, P=0.11) or CD+SSA (6.8% vs. 2.3%, P=0.20) after matching.ConclusionsSSA rarely affects IBD patients, but when it does, it is associated with increased rates of infection, severe sepsis, and multi-organ system involvement. Despite this, SSA does not affect in-hospital mortality in IBD patients. Further studies are needed to explore this association.

Secondary amyloidosis complication of Crohn disease treated with infliximab

Secondary systemic (AA) amyloidosis is a severe complication of progressed Crohn disease (CD) characterized by the deposition of amyloid A in body organs and tissues. Various therapeutic approaches have been recommended, however there is still no effective treatment. Recently, several case reports have demonstrated the effects of anti-tumor necrosis factor-α therapy in patients with AA amyloidosis associated with CD. We report on a 35-year-old female patient with CD complicated by AA amyloidosis in the gastrointestinal tract and renal involvement, who was treated with infliximab. The infliximab therapy improved the gastrointestinal symptoms and decreased the serum creatinine.

Infliximab and Colchicine Therapy in the Treatment of Systemic Secondary Amyloidosis in Crohn’s Disease

Introduction: Systemic secondary (AA) amyloidosis is a rare complication of long-standing Crohn’s disease (CD) with an incidence of 0.9-3%. At present, no established standard of treatment exists for AA amyloidosis in CD. We present a case of AA amyloidosis with renal and colonic involvement in a patient with CD who experienced both improvement in renal function and complete histologic resolution of colonic amyloidosis after infliximab (IFX) and colchicine therapy. Case Report: A 50-year-old male with refractory CD on 5-aminosalicylic acid, 6-mercaptopurine, and chronic steroid therapy was noted to have acute onset anasarca in the fall of 2007. Laboratory studies revealed serum creatinine of 1.2 mg/dL, albumin of 1.3 gm/dL, and proteinuria of 8,258 mg over 24 hours, consistent with nephrotic syndrome. Renal biopsy was performed, and immunohistochemical staining was positive for serum amyloid A, confirming the diagnosis of AA amyloidosis. The patient was subsequently started on IFX and colchicine therapy. In 3 months, he noted significant clinical improvement in gastrointestinal symptoms and was able to wean off of steroids; however, he continued to have nephrotic-range proteinuria. IFX and colchicine therapy was continued with routine follow-up. Gradually, the patient’s anasarca resolved and his renal function was stable, with only trace protein on urinalysis. A colonoscopy with biopsy in July 2009 revealed chronic active colitis with amyloidosis of the rectum, despite a long period of clinical remission. At this time, the patient was continued on IFX and colchicine. He underwent repeat colonoscopy with biopsy in November 2011, which revealed colonic inflammation, but no evidence of rectal amyloidosis. Another colonoscopy in 2012 again showed colonic inflammation with complete resolution of amyloidosis in the rectum. Given the patient’s overall state of clinical remission and stable renal function, colchicine was subsequently discontinued and he was maintained on IFX therapy alone. Discussion: Few case reports have documented clinical improvement of AA amyloidosis in CD patients receiving IFX; however, complete histologic resolution of gastrointestinal amyloidosis after IFX and colchicine therapy has not been previously reported in the literature. It is unclear if this finding occurs in other affected organ systems. Further research is necessary to identify this phenomenon, which has important implications in the future medical management of AA amyloidosis in CD patients. Additionally, AA amyloidosis is a serious complication; routine histopathologic evaluation of bowel biopsy specimen may aid in early diagnoses and treatment of gastrointestinal amyloidosis, as well as assess response to therapy.

Thorough Investigation of a Canine Autoinflammatory Disease (AID) Confirms One Main Risk Locus and Suggests a Modifier Locus for Amyloidosis

Autoinflammatory disease (AID) manifests from the dysregulation of the innate immune system and is characterised by systemic and persistent inflammation. Clinical heterogeneity leads to patients presenting with one or a spectrum of phenotypic signs, leading to difficult diagnoses in the absence of a clear genetic cause. We used separate genome-wide SNP analyses to investigate five signs of AID (recurrent fever, arthritis, breed specific secondary dermatitis, otitis and systemic reactive amyloidosis) in a canine comparative model, the pure bred Chinese Shar-Pei. Analysis of 255 DNA samples revealed a shared locus on chromosome 13 spanning two peaks of association. A three-marker haplotype based on the most significant SNP (p<2.6×10−8) from each analysis showed that one haplotypic pair (H13-11) was present in the majority of AID individuals, implicating this as a shared risk factor for all phenotypes. We also noted that a genetic signature (F ST) distinguishing the phenotypic extremes of the breed specific Chinese Shar-Pei thick and wrinkled skin, flanked the chromosome 13 AID locus; suggesting that breed development and differentiation has played a parallel role in the genetics of breed fitness. Intriguingly, a potential modifier locus for amyloidosis was revealed on chromosome 14, and an investigation of candidate genes from both this and the chromosome 13 regions revealed significant (p<0.05) renal differential expression in four genes previously implicated in kidney or immune health (AOAH, ELMO1, HAS2 and IL6). These results illustrate that phenotypic heterogeneity need not be a reflection of genetic heterogeneity, and that genetic modifiers of disease could be masked if syndromes were not first considered as individual clinical signs and then as a sum of their component parts.

Systemic Secondary Amyloidosis in a 70-Year-Old Patient with Crohnʼs Disease

Systemic Secondary Amyloidosis in a 70-Year-Old Patient with Crohnʼs Disease

Incidence and survival in non-hereditary amyloidosis in Sweden

BackgroundAmyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics.MethodsAmyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008.ResultsThe incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years. However, as rheumatoid arthritis deaths also occurred in other diagnostic subtypes, the incidence of secondary systemic amyloidosis was likely to be about 2.0 per million. The median survival of patients with organ-limited amyloidosis was 6 years. Most myeloma deaths occurred in patients diagnosed with unspecified or ‘other’ amyloidosis. These subtypes probably accounted for most of immunoglobulin light chain (AL) amyloidosis cases; the median survival time was 3 years.ConclusionsThe present diagnostic categorization cannot single out AL amyloidosis in the Swedish discharge data but, by extrapolation from myeloma cases, an incidence of 3.2 per million could be ascribed to AL amyloidosis. Similarly, based on rheumatoid arthritis death rates, an incidence of 2.0 could be ascribed to secondary systemic amyloidosis.

Rapidly Progressive Thyroid Mass

Amyloidosis is a disease of unknown etiology characterized by the accumulation of an amorphous proteinaceous material in various organs and tissues of the body. Amyloid goiter is an exceedingly rare pathologic condition due to massive amyloid infiltration of the thyroid tissue. Amyloid goiter occurs in association with both primary and secondary systemic amyloidosis, more commonly in the latter. Preoperatively, it simulates a multinodular goiter, and surgical intervention is often necessary to establish a diagnosis and to relieve compressive symptoms of a neck mass. We present the case of an 85-year-old female patient who presented with a rapidly enlarging goiter. Histologic examination confirmed amyloid goiter.

Systemic Secondary Amyloidosis in a 70-Year Old Patient with Crohnʼs Disease: Treatment with Oral Budesonide

Purpose: Systemic secondary amyloidosis is a rare but potentially lethal complication of Crohn's disease (CD). It was reported that renal involvement was the most common manifestation in approximately 1% of patients with CD. A variety of therapeutic modalities have been reported, including azathioprine, colchicine, dimethyl sulfoxide, elemental diets and anti-tumor necrosis factor-alpha agents. However, there is still no definitive treatment for secondary amyloidosis with CD. Methods: This is a retrospective chart review. Patient's clinical visit note, procedure and pathology results were reviewed. Clinical Case: A 70-year-old man was diagnosed with colonic CD complicated with a history of perianal fistula since the age of 20 non intermittent prednisone and 5-ASAs. He was also found to have systemic secondary amyloidosis on the kidney biopsy two years ago due to hypertension and elevated creatinine. He was referred to our IBD Clinic in late 2009 for watery diarrhea 10 times per day and bloating. The first colonoscopy showed segmental colitis with edema and multiple colonic strictures distributed in ascending, transverse, descending and sigmoid colons which required endoscopic balloon dilatation. Ileocecal valve was significantly deformed, and not traversible via a pediatric colonoscope. Biopsy showed perivascular amyloidosis on the background of chronic active colitis in all colonic and rectal samples (Figure 1). He had an unsuccessful trial of colchicine from his nephrologist due to adverse effects, and decided not to use immunomodulators and anti-TNF alpha agents due to the concern “malignant potential”. Instead, he was treated with oral budesonide 3mg TID and oral mesalamine 1 gram BID. A repeat colonoscopy three months later showed no active disease, but mucosal scars, and mild active ileitis. Biopsy showed chronic active inflammation in the terminal ileum and rectum with resolution of mucosal amyloid deposits. Clinically patient was doing well on the regimen regarding the frequency of his bowel movements. Serum creatinine and proteinuria have been stabilized.Figure 1: Congo red stain of the colon (A) and rectal (B) biopsy demonstrates submucosal perivascular amyloid deposit (orange colored staining) [congo red, X100].Conclusion: We believe that oral budesonide together with oral 5-ASA agent may be considered a part of medical treatment armamentaria for secondary systemic amyloidosis of CD.

Effects of secondary amyloidosis on arteriovenous hemodialysis fistula outcomes and intradialytic hypotension: A case‐control study

Amyloid fibrils can affect vascular structure through deposition and by causing nitric oxide depletion and increase of asymmetric dimethyl arginine. Patients with amyloidosis are prone to development of hypotension. Hypotension may also affect the maturation of arteriovenous fistula (AVF) and may set the stage for formation of thrombosis and fistula failure. Thus, we aimed to evaluate effects of secondary amyloidosis on AVF outcomes and intradialytic hypotension. This is a case-control study which included 20 hemodialysis patients with amyloidosis and 20 hemodialysis patients without amyloidosis as control group. All patients underwent Doppler ultrasound of AVF. A thorough fistula history and baseline laboratory values along with episodes of intradialytic hypotension and blood pressure measurements were recorded. There was no difference between the groups regarding age, gender, body mass index, presence of comorbidities, hypertension, and drug use. Systolic and diastolic blood pressures were similar (119 ± 28/75 ± 17 and 120 ± 14/75 ± 10 mmHg for patients with and without amyloidosis, respectively). Intradialytic hypotension episodes were also similar. Patients with amyloidosis had significantly lower serum albumin and higher C-reactive protein values compared to control hemodialysis patients. AVF sites and total number of created fistulas were similar in both groups. Flow rates of current functional AVFs were not different between the groups (1084 ± 875 and 845 ± 466 mL/minute for patients with and without amyloidosis, respectively, p:0.67). Patency duration of first AVF was not different between the groups. Clinical fistula outcomes and rate of intradialytic hypotension episodes were not significantly different between patients with and without secondary systemic amyloidosis.

Amyloidosis cutis dyschromica in two female siblings: cases report

BackgroundCutaneous amyloidosis has been classified into primary cutaneous amyloidosis (PCA, OMIM #105250), secondary cutaneous amyloidosis and systemic cutaneous amyloidosis. PCA is the deposition of amyloid in previously apparent normal skin without systemic involvement. Amyloidosis cutis dyschromica (ACD) is a rare distinct type of PCA. Here, the unique clinical and histological findings of two Chinese female siblings with ACD were described.Cases presentationsPatient 1 was a 34-year-old female, presented with mildly pruritic, diffuse mottled hyperpigmentation and hypopigmentation. The lesions involved all over the body since she was 10 years old. There were a few itchy blisters appearing on her arms, lower legs and truck, especially on the sun-exposed areas in summer. Some hypopigmented macules presented with slight atrophy. Patient 2 was 39-year-old, the elder sister of patient 1. She had similar skin lesions since the same age as the former. The atrophy and blisters on the skin of the patient with amyloidosis cutis dyschromica have not been described in previous literature. Histological examinations of the skin biopsies taken from both patients revealed amyloid deposits in the whole papillary dermis. Depending on the histological assessment, the two cases were diagnosed as amyloidosis cutis dyschromica.ConclusionThe two cases suggest that the atrophy and blisters may be the uncommon manifestations of amyloidosis cutis dyschromica. It alerts clinicians to consider the possibility of ACD when meeting patients with cutaneous dyschromia. Skin biopsy is essential and family consultation of genetic investigation is very important in such cases.

When urine is no longer beneficial: renal artery embolisation in severe nephrotic syndrome secondary to amyloidosis

Renal artery embolisation (RAE) is an interventional radiology procedure which is used to embolise small branches of renal artery. It is mainly used for urologic purposes, i.e., vascular malformations, angiomyolipomas and renal tumours not amenable to surgical resection. Bilateral RAE can be performed via using absolute ethanol, polyvinyl alcohol or microparticles. After RAE, patients may experience post-embolisation syndrome which is usually self-limited. Use of this procedure for refractory nephrotic syndrome has been rarely defined in the literature to date. Here, we describe a patient who had nephrotic syndrome due to secondary systemic amyloidosis. The patient presented with severe proteinuria (33 g per day), hypoalbuminaemia and anasarca oedema. We applied bilateral RAE with microparticles. We did not observe any complications associated with the procedure. Protein excretion, laboratory values and clinical signs returned to normal.

Chronic necrotising pulmonary aspergillosis in a marijuana addict: a new cause of amyloidosis

Sir,Secondary systemic amyloidosis has been associated with many chronic infections. Tuberculosis is one of the most common infective causes, and other infections like leprosy, syphilis, bronchiect...